Visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus

Deaner, Jordan D.; Zeft, Andrew; Emami-Naeini, Parisa; Lowder, Careen Y.

doi:10.1016/j.ajoc.2020.100763

Cited by 5 publications

(7 citation statements)

References 19 publications

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“…(APLS) and poor visual prognosis. [1][2][3][4][5] The highlights of our case include the unilaterality and the only manifestation of SLE despite deranged serological markers; the rare presentation of vaso-occlusive retinopathy in the absence of APLS and good visual recovery with vascular reperfusion documented by multimodal imaging with early and aggressive immunomodulatory therapy. Studies have highlighted that ocular involvement can precede systemic features rarely.…”

Section: Discussionmentioning

confidence: 81%

“…They are associated with poor prognosis. Deaner JD et al 5 have shown good visual recovery and vascular reperfusion in a case with vasoocclusive therapy with APLS with aggressive immunosuppressor therapy and blood thinners. Our case though not associated with APLS showed good visual recovery and vascular reperfusion as shown by the multimodal imaging with early intervention.…”

Section: Discussionmentioning

confidence: 94%

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Visual Recovery And Vacular Reperfusion Documented With Multimodal Imaging In A Unilateral Vasoocclusive Retinopathy With Retinal Vasculitis Presenting As The Initial And Only Manifestation Of Systemic Lupus Erythematosus

Babu¹,

Konana²,

Mehrotra³

et al. 2022

Preprint

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Aim: To report a rare case of visual recovery and vascular reperfusion in a unilateral vasoocclusive retinopathy and retinal vasculitis presenting as the initial and only manifestation of systemic lupus erythematosus(SLE) despite elevated serological markers. Materials & Methods: Retrospective case report Results: A 33-year-old lady with no systemic complaints, presented with blurred vision in left eye(OS). She was diagnosed to have unilateral vasoocclusive retinopathy with retinal vasculitis and macular edema(OS). Fluorescein angiography and OCTA confirmed areas of multiple arteriolar occlusions(OS). She was diagnosed to have SLE on the basis of strongly positive antinuclear antibody, nRNP/Sm(3+), SS-A(2+) and Rib.Pprotein(2+), leucopenia(3400cells/mm3), increased ESR(68mm/hr) and low complement-3 levels. There were no other clinical signs of SLE. Antiphospholipid antibodies were negative. Treatment included pulse doses of intravenous methyl prednisolone followed by oral steroids, pulse doses of intravenous cyclophosphamide, posterior subtenon steroid injection and sectoral laser photocoagulation. Serial multimodal imaging showed vascular reperfusion and good visual outcome. Conclusion: This case report highlights the excellent visual outcome and vascular reperfusion documented with multimodal imaging in a rare case of unilateral vasoocclusive retinopathy and retinal vasculitis occurring as an isolated finding in SLE despite elevated serum markers. These findings suggest that the vaso-occlusive disease is reversible if the diagnosis is made promptly and intensive therapy is initiated.

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Section: Discussionmentioning

confidence: 81%

Section: Discussionmentioning

confidence: 94%

Visual Recovery And Vacular Reperfusion Documented With Multimodal Imaging In A Unilateral Vasoocclusive Retinopathy With Retinal Vasculitis Presenting As The Initial And Only Manifestation Of Systemic Lupus Erythematosus

Babu¹,

Konana²,

Mehrotra³

et al. 2022

Preprint

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“…Most of the patients had poor visual prognosis, and 9 out of 11 patients had CNS manifestation probably reflecting a similar pathogenetic mechanism. On reviewing various case reports 6,[9][10][11][12][13][14][15] of CCRAVO in SLE patients with secondary APS, we found that few had bilateral severe vaso-occlusive disease 10,13,14 but most had unilateral presentation with the unaffected eye being normal 6,9,11,12,15 but none had optic nerve-related manifestation. 6,[9][10][11][12][13][14][15]27 Pathological study of blood vessels in CNS lupus and vaso-occlusive retinopathy disease shows thrombotic vascular occlusion rather than a vasculitis.…”

Section: Discussionmentioning

confidence: 88%

“…[6][7][8] Vaso-occlusive form of retinopathy occurring in SLE patients with positive aPL is associated with poor visual prognosis. 6,[9][10][11][12][13][14][15] The diagnosis of APS is based on Sydney criteria 7,16 which includes the occurrence of vascular thrombo-embolic events or pregnancy morbidities, and the presence of these antibodies on two or more plasma samples taken at least 12 weeks apart. 7,16 In patients with both SLE and APS, the pathogenesis of severe vaso-occlusive retinopathy is attributed to thrombosis associated with APS, rather than the immune complex-mediated vasculitis related to SLE.…”

Section: Introductionmentioning

confidence: 99%

“…10 We report a rare simultaneous presentation of complete combined occlusion of central retinal artery and vein (CCRAVO) in right eye (RE) and optic neuritis in left eye (LE). There are very few case reports 6,[9][10][11][12][13][14][15] with CCRAVO due to secondary APS in SLE patients, and in most of the case reports the other eye was either normal 6,9,11,12,15 or had evidence of bilateral severe vaso-occlusive retinopathy, 10,13,14 but none had optic nerve-related manifestation. To the best of our knowledge, this is the first case report with such simultaneous presentation.…”

Section: Introductionmentioning

confidence: 99%

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A rare simultaneous presentation of combined occlusion and optic neuritis in a lupus erythematosus patient with anti-phospholipid antibody syndrome

Prakash

Gunderia

Khadar

2023

Lupus

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Background A known case of Systemic Lupus Erythematosus (SLE) developed sudden loss of vision (no light perception) with relative afferent pupillary defect in her right eye (RE) and fundus examination revealed cherry red spot over tomato splash background with tortuous and dilated veins suggestive of combined central retinal arterial and vein occlusion. Analysis Left eye (LE) had 6/6 vision but posterior segment revealed a hyperemic disc. Fundus fluorescein angiography (FFA) revealed delayed arm to retina time and increased arterial to vein transit time in RE. LE showed pooling of dye around optic disc suggestive of SLE related Optic neuritis. Patient was re-evaluated and she tested positive for Lupus anticoagulant. Patient was diagnosed to have anti-phospholipid antibody syndrome (APS) occurring secondary to SLE. She was started on intra venous steroids, anti-coagulants and disease progression in left eye was controlled. Results We report a rare simultaneous presentation of complete combined occlusion of central retinal artery and vein (CCRAVO) in RE and Optic neuritis in LE which has not been reported in literature so far. Conclusion Ocular involvement may precede other systemic symptoms in SLE, so ophthalmologist should know about this rare simultaneous presentation. Ocular activity can correlate with systemic disease activity and starting early effective treatment can prevent other systemic lesion and can be life saving.

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Risks of posterior segment ocular ischaemic events in patients with systemic lupus erythematosus: a population-based cohort study in Taiwan

Chiou

Chang

et al. 2022

Br J Ophthalmol

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Backgroud/AimOcular involvement in systemic lupus erythematosus (SLE) is often primarily recognised by ophthalmologists rather than internists. This study aims to investigate the incidence and risk factors for the occurrence of posterior ocular ischaemic events (OIE), including retinal vein occlusion (RVO), retinal artery occlusion (RAO) and ischaemic optic neuropathy (ION), in patients with SLE.MethodsA national database in Taiwan was used to identify 24 472 patients newly diagnosed with SLE and 244 720 age-matched and sex-matched controls between 1997 and 2012. New occurrences of OIE and confounding factors were recorded. The Kaplan-Meier method was used to compare the risk of OIE between the two groups. Fixed effect models were applied to evaluate the risk factors for OIE.ResultsThe mean age was 36.24±15.82 years and women accounted for 88.4%. Patients with SLE had significantly increased risk of overall OIE (HR 3.89, 95% CI 3.36 to 4.50, p<0.001) as well as each OIE subtype. End-stage renal disease (ESRD; HR 2.91, 95% CI 2.05 to 4.14, p<0.001), hypertension (HR 1.77, 95% CI 1.21 to 2.58, p=0.003) and congestive heart failure (HR 1.67, 95% CI 1.12 to 2.48, p=0.01) were associated with RVO development. Hypertension (HR 2.89, 95% CI 1.10 to 3.96, p=0.02) and ischaemic stroke (HR 3.58, 95% CI 1.97 to 6.48, p<0.001) had increased risk of RAO. ESRD was associated with ION (HR 3.03, 95% CI 1.41 to 6.51, p=0.004). Intravenous steroid was associated with RVO development (HR 2.54, 95% CI 1.67 to 3.84, p<0.001).ConclusionsSLE increases the risk of developing OIE. Systemic comorbidities and higher dosage of steroid in patients with SLE are associated with severe ocular ischaemic complications.

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Visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus

Cited by 5 publications

References 19 publications

Visual Recovery And Vacular Reperfusion Documented With Multimodal Imaging In A Unilateral Vasoocclusive Retinopathy With Retinal Vasculitis Presenting As The Initial And Only Manifestation Of Systemic Lupus Erythematosus

Visual Recovery And Vacular Reperfusion Documented With Multimodal Imaging In A Unilateral Vasoocclusive Retinopathy With Retinal Vasculitis Presenting As The Initial And Only Manifestation Of Systemic Lupus Erythematosus

A rare simultaneous presentation of combined occlusion and optic neuritis in a lupus erythematosus patient with anti-phospholipid antibody syndrome

Risks of posterior segment ocular ischaemic events in patients with systemic lupus erythematosus: a population-based cohort study in Taiwan

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